• I .

. '"

Congenital anomalies

In

Neurosurgery

•

..

1. Hydrocephalus

Definition :

L(iltJ~'l1tJb'Wl'llJ'il~tJ~:;n'il1JIif'ltJ~'l'WtJ~:;n'il1J~1r1~3 ~'l'WA'il••• 1 •1. Cerebral parenchyma 'l~u~:;n'il1J(iI'ltJneural LLI'l:; glial all

2. Vascular system ;~~(ilIii'ilri1Jll1tJ'W'ilnL(iltJeJ1'W'II1~Carotid LLI'l:; vertebral arteries LLI'l:;

jugular vein

3. Fluids tJ~:;n'il1JIif'ltJCSF LLI'l:; extracellular fluid ~~1'l~1~LLI'l:;~(iI~lJni1J'II1~vascular

system

L(iltJn1~LLtJ~LL1J1J~hydrocephalus t.llJ1tJ~~n1~LtJ~tJ'WLLl.Jl'l~'II1~Hydrodynamic '1J'1I~

CSF ;~vi1h\Ln(iln1n~lJmlJ1ru '1J'1I~CSF b'WCNS

Incidence:

er~1lJ"W11Jincidence ~LLl.ii(il LLIiii:JlitJ~:;lJ1ruH~1Vi1JtJ~:;lJ1ru3 l'1'Wb'WL~mn(ilbt.llJ•1,000 1'1'WLLI'l:;b'WtJ~:;LM~~00-I'W1LLI1'l,,:;i:J"llliin1nu'1J'il~L~nHydrocephalus 11ifl"1Jn1~l"m,.1

tJ~:;lJ1ru 15,000 ~~ 25,000 nulii'1ltl

Physiology of CSF

L(iltJl.Jn~ CSF 1'l~1~tIf'ltJn~:;1J'l'Wn1~ultrafiltrate r.i1'W'II1~nontight junction '1J'1I~

endothelial wall '1J'1I~choroid plexus lif'ltJtllil~1tJ~:;lJ1ru 21 cc/hr. ~'l'W~'W'lJ'1I~l'llJ'1I~~Vi1t.li.h

~1'l~1~CSF nA'1I ependyma L(iltJ":;1'l~1~"1nchoroid plexus 1.J~:;lJ1ru80%.• Q" , :'1n1~~(iI'llJ'lJ'1I~CSF ":;Ln(il'll arachnoid villi '1J'1I~venous sinus \(iltJLu'WLL1J1Jpassive

absorption ~'Wri1JI'1'l1lJLLlilnIii1~'lJ'1I~1'1'l1lJ~'Wb'Wsubarochnoid space LLI'l:; sinus•.• -CSF hydrostatic pressure '1J'Wn1J Active secretion '1J'1I~CSF

Resistance to CSF flow

Passive resorption in venous system.

b'W0-t.lqjb'W'l'11'W'1I'W,,:;i:J ICP 1.J~:;lJ1ru122:2 cm H20 L(iltJb'Wnewborn LLI'l:; infant

Pathophysiology

Hydrocephalus Liil1l~1nn~'ln 3 'IIUI1I~'il

1. Overproduction of CSF

Choroid plexus tumor

Hypervitamnosid

2. Increase resistance to CSF flow

Communicating

Non communicating

Pathology - Malformation ( Aqueductal stenosis, chiari malformation)

- Mass lesion (tumor, hematoma, cyst)

- Inflammatory ( infection, hemorrhage)

3. Increase in venous sinus pressure

Organic lesion ( sinus thrombosis, tumor)

Functional origin (AVM)

Etiology :q .l' 1 ,~ r

1. Prenatal Cause : Lnl1l'1l'W'Wr::"'~1~I1I~ArrJl

- Aqueductal stenosis dve to malformation vnJ1.h::mfW 10% '1I'il~hydrocephalus

1'Wnewborn LLU~Li'I'W3 'IIUI1I~'il

Aqueductaf Stenosis

2

: Aqueductal gliosis

forking of aqueduct

Ependymal septum

Dandy - Walker Malformation

Dandy - Walker malformation

- Arnold chiari Malformation ( type II )

- In Utero infectiono

Congenital toxoplasmasidAI

Cytomegdo virus

- Destructive cerebral lesion of ischemic origin

- Familial hydrocephalus

: Bickers Adams syndrome

3

~~'1l~~~l-J1A'1lArachnoid cyst, spinal tumor

4

2. Post natal course

- Mass lesion

Choroid plexus tumor

~u 20% 'IJ'1l~hydrocephalus 1m~n ci~lJ1"'C1idJlJposterior fossa tumor L'IIlJ

Astrocytoma, ependymone medulloblastoma

- Hemorrhage

Lnl1l"l1n\\1L"'liJlih~1 nlJ L'lilJPrematurity, AVM , trauma 1lJ acute stage "161 Fibrin•

LL~:;clot hJ'1JI1Iv1lJVJ1~L~lJ,j11'JJli'lJmr~LLlii1lJchronic stage Lnl1l"l1nArachnoid fibrosis

- Meningitis

Bacterial meningitis -.111~Lnl1lArachnoid granulation LL~:;inflammatory

aqueductal stenosis

TB meningitis "I:;Lnl1larrchrorditis 'IJ'1l~basal cistern

- Hydrocephalus of venous origin

Lnl1l"l1nanatomical ",1'1lfunctional blockage vh1~ venous pressure L~l-J~lJ

- Idiopathic Meningitis

Clinical Aspect

~lJnU'1l1U'll'1l~L~n1lJ infant '1l1n1~~~ulriil.j'1lu ~\\111ii'1l L~n1X~t(;l~I1I1.ln~, ,

Macrocrania "'l-J1Ui\~ ~'1lu1i1~~:;l-J1nn~1 2 SO ",1'1l98 percentile LL~:;'1l1"1"1:;~U~1iJn1~

h1~iii~'lI'1l~anterior fontanelle, d~lJ",1'1lA~1~U~1 suture LLtln,mr~1i1~~:;U1~1\\ LL~:;L~lJ vein

- , ~'Il(;l,setting - sun eye phenomenon, r;)~1•• eye gound 'El1••'>1U11l-Jvenous engorgement

\11'ilearly optic atrophy

1t.lL~n~ fontanelle U(;l ••:;'>1ui1'El1n1~'lJ'il~intracranial hypertension L'Il'Uu1~i'lat:;

'il1L~U'U~l-JiN sign '1J'il~brain stem dysfunction

L'Umru'lJ'il~Chronic intracranial hypertension lTn••:;LLi'I(;l~'El1n1~L'UL~nL'>1~1:;L'U'Il1~

vmn n~:;Lm\ni'l~1:t:; ••:;'lJU1u1~Ll1in••:;i1.J1Lr;)L'U'Il1~LLm.l'ill-J1••:;i114'1ilJ'U1n1~~(1IUn~~1

suture L1l-JU(;lL~n••:;i1'El1n1~U1(;li'l~1:t:;'El1L~U'Ui1fl11l-J~1LLi\:;'>1~~m~l-JLU~U'ULLUi\~

Diagnosis :

- History and clinical data

- Neuroradiological examination

Tran fontanellar ultrasound

hjI\1l-J1~tlU'ilnI\1L\11il'lJ'il~hydrocephalus 1ar

Computed tomographyI . 4 .

1\1l.J1~tlU'ilm1 ventncle \1~'ilsubarrchold space chililation

periventricular edema i'l1L\1Iil'lJ'il~ohstruction 1ar

: MRI

1\1l-J1~tl(;lfl'l1l-JCj(;lUn~'lJ'il~hydrodynamic 1ar"nn CSF flow pattern..Treatment :

1. Temporary treatment

Medical treatment :

: i\(;ln1~1\l'1~CSF ( Azetazolamide , Furosamide )

: L~l-Jn1~r:J(;l~l-J( Isosorbide )

- External drainage :

Ventriculostomy

Lumbar drainage

2. Alternative to shunting

- Etiologic treatment

Ltl'U1fin1~fm~1~~~I\~~1\1fu hydrocephalus L'Il'Ucontrol vitamin A,, . ,

.c:!l ••••• '" , .,.

intoxication, Radical mass resection 'Il~U1~m~'El1"":;r;)'il~n1~ temporary drainage n'il'UYl

.•:;vhn1~fn1:t1i'l1L\11il'lJ'il~hydroceshalus

5

6

- Membrane Fenestration

H1:wmruiJn1~'1JIn~l.I ~ Aqueduct of Sylvius ,l~'il 4" ventricle "'1tInEJn1~, , .

V'l1:; floor 'JI'il~3'd ventricle Li1'ilLtI~EJl.In1n~1.I'111~'JI'il~":111'J1i\l.I'lo1~~hJ!T~subarachnoid

space

3. Shunts

fl'iln1~l'1r1~'111~i'iInIii'il~:;'lo1~1~CSF 11.1ventricle 'lo1j:'illumbar subarachnouid space

1t1!T~drainage cavity L'lil.lperitoneam , right atrium, pleural cavity ..• , • ~ • '..I 1~ •• J''JI~11.ILlnnl.lEll.J'II1VP shunt L~~1:;1'11l-J1~(l1'~1'11EJ1nEJ11mn:;Llnn"l:;\11I'J11.1LL~:;f'111l.1

Shunt complication

- shunt infection

- shunt occlusion

- Disconnection

- Improper placement

- Over drainage

2. Encephaloele

fl'il cystic congenital malformation ~~iJ'1I11.1'J1'il~CNS ~Iii'ilnu CSF pathway ~1.I~11.1

~'ilmLEJn'JI'il~n~:;t'lo1~nF'h~:;'il"Jnl-J1~1iJL'il~1:; meninges nu CSF L~EJn~1meningocele LLIii

'" ..::01 . "'.::ol'.

ml.l Neural tissue 'il'ilnl.l11n1EJ"l:;L~EJm1meningoencephalocele

Classification :

CLASSIFICATION OF ENCEPHAlOCELES

8y StructureMt"J\ingl1l(l:"1t"Meningoencephalorek"

By LlX.ltionPosterior

Supra torcularlnfratorcular

Anb!riorFnmtalSincipitltlNilSO£rontalNa~fhmojd.J1Na50-orbital .

BasalT ransethmoidallr.1n.~sphenoidal

7

Embryology :

L~'1l,:hEncephalocele d:J'Ufl11l-1i1(;1t1niii~L1i(;l~i\'~"l1nneural tube 1I1il1(;11t1LL<11

(day 25 ) L~n::L~I'J~1 Cerebral cortex ~j-'1lCerebellum "l::1'l~1~~i\'~"l1n neural tube u(;I1t1

LL<11L(;ItJ1:r"l,u'Uu~1lJVI~1t1mechanism ~";11~L1i(;lLLIiiL;'1l~1CNS strnctum LfI~'1l'U~~1'U

mesenchyme defect L~'1l8 ii~12 wks '11'1l~gestation

Incidence:

~tltI~::l-I1ru 1 ntJlii'1l L~n~L1i(;l1mJ5,000 ii~10,000 ~1tJL(;ItJVl1~r;l::i'Ur;ln"l::~tld:J'U

posterior type t1~:::l-I1ru% LLIiiVl1~Senthern Asia lJn~tld:J'U anterior type

L(;ItJ'iir;ln\'h'U'1I'1l~L~PI'l1tJLvi11 ritlL~PI~~~ 1'Uanterior type LLIiiposterior type "l::

~tl1'UL~PI~~~mnn~1 \'h'U1~qJLtJ'U sporadic case

Diagnosis evaluation :

tJnL-1'Uoccult basal lesion tI1~mh~ ri1'U1~qJr'i'1l'U"l::L~'U1Jfi(;ldiagnostic study of

choice ~'1lMRI ~j-'1lMRA chi'J vascular structure '1l~ilhtJ 1'Unn1j'1l'1l~sincipital ~j-'1l basal

encephalocele m"l"l::tlI'1l~";1 CT 3 dimension L~'1l1-n'Un1~";1creniofacial reconstruction

1'Un~nh\'11'l~~tJhydrocephalus m"l"l::iii(;lr;l1l-1L(;ItJ1-ifultrasound ~j-'1lCT Brain ':J

In utero detection

L(;ItJ1-iffetal ultrasound LLI'l::L"l1:: blood AFP '1I'1l~m~(;I1':Jd:J'Un1~screaming

- Remove sac

- Preserve functional. neural tissue

- Closure with non dysplastic skin

8

Posterior Encephalocele

Prognosis ~i1'~n11~11il~~U'1l~nu'1J"'1~'lJ'1l~encephalocele tIhl1ru'lJ'1l~ neural

tissue 1•••sac, degree '11'1l~microcephaly 1•••n1ru~tIhl1ru neural tissue 1•••sac l-l1nn11

1•••m:: 'lm~nPlnl:: '1l1"lhi~1 d'l•••.,'1l~~11il~ LLm'11~~.Jf1~1mm~nnu~t1nf11'1l~L~nri'1l'"LL.J1•••n1ru•meningoele f1~nhlil~'IInntJ•

Posterior Encephalocele

Anterior Encephalocele

"l::LLliln.J1~"l1nposterior type L'Yi11::11neurologic function lJm::t1n~ f1'll-llil~tJ

lj~mr~t1n~ hifi'1ltJ'YiUhydrocephalus

Anterior Encephalocele

9

3. Arachoid cysts

Definition :••• .o:!I. col.,. J' 'I

Primary 'MtJ congenital arrachnoid cyst AtJfluid collection Y1Lnlil'll.lb\.l

". .•. , ••..arrachnOid membrane ~1nn1mEJn"'1tJLLtJ~'D\.l'lltJ~arrachnoid membrane

'[~EJ~~hIL~tJ~1LU\.lm1~ LU\.l~~t1niiiLL.JrhLij~ L'fIn:;'fItJ~1Ln~L\.lL~nLL'i'l:;0my L'Ih1" .

ti\.l tJ1~Af~'fItJL\.lA1tJtJAf~L~mn\.l'fItJi~~nl.Janomalies ~\.l1 L'Ii\.labsence of sylvian vein

agenesis of corpus collosum

n'i'l1nL~mntJn1nn~ET~hiYl11tJLLUi~ LL.JL:ntJ~1Ln~~1nn111~~til~primitive

mesenchyme ",1'lILU\.lA~1~~~t1niii'lltJ~flow 'lItJ~CSF ~~":hL1XLn~subarachnoid space L\.l

'Ii~~LLm'lltJ~embryo.

ANATOMICAL CLASSmCATION OF ARACHNOIDCYSTS

Intracranial CystsE.lradural inlradiploic cysh;Supral'''TltorialIn(ral.enillrial

Intradural cystsSUpr.llC'ntorialSylvianSellar and supr.J~l'lIarCerebral convexityInterhemisphl'ricalIntraventricular

Optic nerveQuadriKeminal plan.lnfratentorialCerebellar hemisphereMidline vermian-<:istl'rna magnaCC'rebelloponline angleIntr ,wl'ntricu IarR"trodival

Spinal CystsExtradural cystsIntradural cysts

'fItJ - 1% 'lItJ~space occupying lesion L\.l~~tJ~i\~\.lLmYLU\.lsporadic LL'i'l:;'fItJLU\.l

single lesion 'fItJL\.lL'fIPI'lI1EJt11:;~1ru2/3 i\~\.lLmY'fItJL\.l'li~~mEj 10 -20 iJ ,rn~ru:;n11~1Lij\.lhA'lItJ~arrochnoid cyst ET~hiYl11tJLLUi!i\tJ1~nEJm~hiihl1n1n!lEJIil!ltJ~mlil tJ1~nEJ

,~ J' :c. 1.'1"AtJEJ1 ~mn1mln'll\.l1J1~ANn"'lEJ u .wen

• •'1I1n1~Ii~'W1~n.J\1i11l"1nn1ml1l\utJl1ILi1'11\'ll-J'1I~~1'11'111~\~'Wu11']J1i'W~~~-l1~\fitJ~

mn1~~~u~'W'1I~nU~1Lmti~~~u cyst \\~'1I1n1~~~uh'hj'1ltJ 1\-D'Wcranial

enlargement,localized cranial bulging, increase ICP , seizure, retardation, focal

neurologic deficit mn1~~'Wl ~m"'r1uli11 L-D'Wendocrine disorder "Bobble head doll"

symdrome

Pathophysiology :

'II~~5~\~'1I~1 arrochnoid cyst t'"~'W"1n'1l:;hil 2 'II~~5 A'1I

1. L~'1I~1arrchniod cyst hnill~'1Inu subarrochnoid space \\liit,"~'W"1n fluid ~. ~M1~"1m.J'W~']J'1I~cyst

2. Ball - valve hypothesis L~'1I~1iln1~~I1IIii'1l~Yl1~U1~Li'i'Wone - way Valve

~:;Wj1~ subarrachnoid space LL\'1:;cyst Yl111XCSF \'l1mnH-l1hJ1'W cyst li11

Investigation :

1i~'W1~n.J';jij"'tltJ"1n CT ~1'11MRI ":;'r1uLi'i'Wwell define cyst ~:;~~1~ dura L\\'1:;

distorted brain L\\'1:;,,:;ildensity miJ'1I'WCSF , non enhanced

10

Arachnoid cyst

nl',i'ml1• Conservative management

:1~nsign 'lJ'il~increase ICP \1l-'il focal neurologic deficit

- Surgical management

: Excision

: Shunting of cyst fluid

11

12

4. Chiari malformation :

iii'tl 1'I~1l-Jijliltlniil'lJ'tl~hind brain ~~"'1I;h~~l-J1~1n~1Forramen magnum

LL1J~LUt.l4 '1IUIiiiii'tl

Caudal displacement of cerebellar tonsil

II Caudal displacement of cerebellar vernis,

Fourth ventricle, lower brain stem

III Caudal displacement of cerebellar, brain stem

IV Cerebellar hypoplasia

Type I Chiari Malformation

Epidemiology :

Yl1Jjj'tl1n1~1••••Ji~~'tl1!J~~LLIii10-70 iJ 1Ii1tJ"l:;Yl1J1t.l~\1t11~l-J1nn~1~'1I1tJ(1.3:1)

~thtJ chiari type I 'tl1"ll-J1i1htJ'tl1n1~'lJ'tl~brain stem compression, hydrocephalus,

sysingomyelia 'tl1n1~~Yl1J1t111JmJ1 L'lit.lu~liIP1nt:; tI~liIf'1'tl'111'!i'tlt.lLLNl-J'tl~L~t.lI11Yl.jf'tlt.l

- Fora magnum compression syndrome (22%)

- Ataxia, sensory deficit, weakness, cerebellar sign, CN palsy

- Central cord syndrome

- Dissociative sensory loss, symental weakness long tract sign, lower cranial nerve

palsy

- Cerebellar syndrome

Evaluation :

Plain film

Skull LL~:;C - spine film Yl1JI'I~1l-Jijliltlniiltl~:;l-J1ru35% L'lilJbasilar impression,

platy basia

13

MRI

Diagnortic test of choice ~:;~1J~1 tonsil ~:;'il~~1n~1 formen magnes > 5 mm. LL\1:;

'il1~~1JjJ syringomyelia i~lJlii~EJ

m,;n""LLU:;U1 t,xvhm~~11iiI11tu~1l~EJ~jJ'il1m~ ••.{j('iljJ'il1m~LL!.J~NVln~1EJLI11EJm~vh• •

porterior fassa decompression

Type II chiari Malformation

'il1~~:;~1JjJm1lJtjl11l1n~ ~u 1i~lJlii~EJL'lIU becking of tectem , abscence of

septum Pellucidum . hydrocephalus, microgyria, bony abnormality

.•nm,Lnl11~1nl'1~1lJtjl11l1n~'lJ'il~brain stem LL\1:; lower eN 'il1m~~Lnl11~utuL~nLi1n~:;LL!.J\1~

L~'ln~1L~n~LIi1LLi\'~~~~~1J1IiiLLrin~U~11J1n. apnic spell, stridor, aspiration, arm weakness

Evaluation :

MRI Li'lu diagnosis of choice

Posterier fossa decompression.

I ..

5. Syringomyelia

Definition

~1~1nc;)m1lJ'JJ"N syringomyelia 'lilriJ0-X~1~1nc;)m1lJH\1~1!J 1~l.JLLl.Jl.JL'liU•,0 0.... •.•• ,

tubular cavitation of spinal cord extending over many segment LLIIlA1"l1nC;)I1'l1lJUI1'i1U. ," 4 •.•.•••• •••• .:lI

'JJ1~"l:;l1f'il1.J11'llJlJ1n L~f1:;\1lJ1!J11'l1lJ[J~ cyst 'VI~\1lJC;)'VILnC;)"l1ntrauma, tumor \1f'il

abnormal 'JJ'iI~ craniovertebral junction ~~'lilriJ~i'\C;)~1~1nc;)m1lJ~L'il~1:;n11 Lc;)ml'lu

Intramedullary cyst with CSF like content ~hJ1'li tumor cyst \11'i1 parasitic cyst I'l'lUd

hydromyelia \1lJ1!J11'l1lJ[J~ distend 'JJ'iI~ central canal, lined by ependymal cells and

contain fluid identical with CSF

Syringomyelia

14

15

Classification

1i11i:i~classified syringomyelia '[11I!J1n'ill;j~~1n pathoetiology, MRI and

pathological information '[l1ImLl.1~LlIu6 'JIUI1If1'il

1. syringomyelia communicating with fourth ventricle ~ul.Jr::lJ1ru 10% cavity lJ'mllu~ ~ .,

central type LL~::n1rrm~1"'iln1rl'l1 shunt

2. syringomyelia due to blockage of CSF circulation of CSF circulation ~lJl.Jr::lJ1ru, .

50% 'IJ'il~case Liil1l~1nn1r block 'IJ'il~CSF circulation ;;;~1Lmll~1I11ri1r,1M~LLIii

posterior fossa ~~11.J~1mlil~~1J1i11L'liuChiarimalformation, basal arachnoiditis,

n1ri"m:nlJ'm::LlIu release adhesion, decompression at level of blockage

3. syringomyelia due to spinal cord injury ~1mliJ'il1~Liil1l~1n post necrotic cavitation,

posthemorrhage ",,;l-'ilmyelomalacia syringe lJ'n"l::LlIu focal LL~::eccentric ~1J

U'il!Jn~1 10%~ ~

4. syringomyelia and spinal dysraphism n1rrm~1"'il untethered and release adhesion

5. syringomyelia die to intramedullary tumor, fluid 1u~1n~lJ'mllu yellowish,

proteinaceous himi:i'ilu CSF lJ'n"l:: secrete ~1n tumor 1.1r'ilLiil1l"l1nCSF

blockage

6. idiopathic syringomyelia ~'iln~lJ~hiL;hrilJ 5 ojf'ilLrl'il~ilfu~~'il1~"l::hi'm1U~1L1.11iJ1.1r'il

Liil1l~1n occult blockage 'IJ'il~CSF circulation

Pathogenesis of syringomyelia

iJ"l"lUul'l~1:l'DL~m rilJn1niil1l'IJ'il~ syringomyelia iJ~.,~1l.il'lr11JLLlli1111i11i:i~~!J1!J1lJ

i11111'1~1:l'DIii1~1 L~'il'ilfi1J1!Jn1rLiil1lsyringomyelia LLliihJi11'1~1:l'D1111~'1lJ1nl'ilfi1J1!Jn1rLnl1l

syringomyelia 1i11i~1.1lJl1I~~ pathogenesis 'IJ'il~syringomyelia ~U'il~riu~1mliJ":h11XLiil1l

1. mechanism for entry of fluid into spinal cord

2. mechanism for expansion of cavities in cord

16

1, Mechanism for entry of fluid into spinal cord

Chiari L~'1l~1Lnl1l'nnpersistent of a physiologic state of embryonic hydromyelia

with hydrocephalus LLliihJtl'\~u'WYjU~1i'l hydrocephalus 1~l-Itl!~mV1u~10%

Gardner; Hydrodynamic theory L~'1l~1Liil1l'\1nintracranial CSF arterial

pulsation force,

CSF cJ1'Wpersistent opening u1mu obex L-ih~ central canal w~:;i'ln1~"lI1l~'WV1outlet

'lI'1l~fourth ventricle LLIii'\1nn1~Mm~I1Iii'1ll-l1yjU~1i'lopening ~ obex U~:;l-I1ru 10% L\'11•u'W LL~:;hi\'l1l-11~n'1lfiU1umru non communicating ttl!

, ,.4, <:>IQ Q

William: Craniospinal pressure dissociation theory L'lI'1l~1n~ruYlLnI1lhind brain

herniation l-I1'1l11l~'W~foramen magnum '\:;'.111,xLnl1lone-way valve '.111,x CSF t1,lm.n1'W•subarachnoid space 1'Wspinal cord ttl! LLliit1,l~ni\'uhjttl!Lri'1lpressure 1'Wsubarachnoid

L~l-I~'Wl-I1nn~1intraspinal pressure '\:;~n~l1I;l-IL-ihhJ1'Wspinal cord

Ball and Dayan L~'1l~11'Wnnw~i'l tonsillar impaction, fluid '\:;L'ihhJ1'W spinal

cord cJ1'WYl1~Virchow Robin space

Aboulher L~'1l~11'Wn~ru~i'lobstruction fluid cJ1'WL-ih1'Wcord Yl1~ dorsal root

Oldfield L~'1l~11'Wmru~i'ln1~"lI1l~'W'lI'1l~foramen magnum, systolic pressure•

wave '\:;~'W111cJ1'WL-ihhJ1'Wspinal cord cJ1'W\'l1~perivascular LL~:; interstitial space

Milhorut L~'1l~1ependymal cell \'l1l-11~n\'l~1~fluid ttl! LL~:;Lri'1li'lependymitis

'.111,xLnl1lobstruction 'lI'1l~CSF upward flow '.111,xLnl'ln1~dilate 'lI'1l~central canal

2., Mechanism for expansion of fluid cavities 1'Wcord

Gardner; Lnl'1'\1n progression dissection 'lI'1l~CSF cJ1'W\'l1~obex L-ii1l-111'W

central canal

Ball and Dayan L~'1l~1n1~ increase accumulation 'lI'1l~fluid 1'Wsyringe cavity

'.111,xi'ln1~LI'I~'1l'WV1'l1'1l~fluid :j1'W\'l1~ parenchyma L-ii11'Wcentral canal

Williams L~'1l~1fluid dissection 1'Wcord Lnl1l'\1n pressure differential '\1nn1~

distension 'lI'1l~epidural vein ~:;1,l~1~~i'ln1~t'1l1,l'l'-'1l'\1l-1d ,

Oldfield L'lI'1l~1systolic pressure wave 'lI'1l~subarachnoid CSF 'lI'1l~

subarachnoid CSF '.111,xLnl1ln1~expansion 'lI'1l~cavity

17

Syringomyelia associated trauma, ,

~ I ••••• QQ ~ ~ ~ oj

L'Ilm1Lnl1l"1nmn'll,J cord injury LLmLnl1lhematoma ",r'1l~ spinal cord necrosis

"1n ischemia ..1111l'Lnl1lmr obstruction 'lI'1l~ CSF

Syringomyelia associated tumor,

L:n'1l~1mechanism 'lI'1l~ syringomyelia Lnl1l"1n obstruction 'lI'1l~ CSF ",j:'1l"1n

edema, blockage of perivascular space cavitation secondary to hemorrhage or autolysis

Diagnosis and clinical presentation

n1r 11l'mr:JU,,'iilJ syringomyelia ,,1 nur:::oWiLL~:::mrMr~"i1~ mlJ m"":::..111I11mn, ,

LVir1 :::~U~lJ\'l1mnll.J1111~lJm mrVlLLMn~h~ri\,l1111~\,lri1J pathology 'lI'1l~mrLnl1ll1l~lJ•\'h\,l1m1!LLI'I'~mnnl1l syringomyelia lJn":::A'1llJ 1Llll1lH'L~~1"'~1lJiJ LL~:::lJn,,:::hJ

""lJ1UL'1l~ 1\,lnnu'll'1l~ syringomyelia ~Lnl1li~~ri1J type I Chiari malformation lJnVi1J1\,l

'li~~m!J 20-40 iJ '1l1mr~Vi1J1I11fi'1l dissociate sensory loss, cervical and occipital pain,

lower motor neuron weakness 'lI'1l~iJ'1lLL~:::LL'lI\,l,neurogenic arthropathies ~U~lJlJn,,:::~~

l1I~lJmn1r'll1~iJ'1l ",j:'1lLL'lI\,lLL1J1Jasymmetry ~l1IlJ":::Lli!JLLIiipain, temperature sensation, '

, Q ~ Q '." Q .IQ .1\l1IlJ'VItouch and joint position lJ~11ILL~:::~u~lJm",,:::~mnu~lJ\,lLLU~~'lI'1l~ neurogenic. ,

•••• , ••••••••• «:lI

finding "'~~,,1n valsava maneuver mmr'1l'1l\,lLLN~m:::Ln,J,,1n fine motor 'lI'1l~LL'lI\,l",r'1l

iJ'1lLL~:::ij atrophy i~~ri1J absence 'lI'1l~ DTR, bowel bladder lJn,,:::un~ LLliim"":::Vi1J

Honer's syndrome 1111

Vi1J~1iJ hydrocephalus ur:::~1ru 10-33% 1\,lnnUVi1Ji~~ri1J type II Chiari«:lI.... «:lI oj

malformation '1l1mr,,:::L"'~'1l\,ln1J type I i1'1l~ spastic paraparesis, lower motor neuron

weakness in upper limb, dissociate sensory loss, developmental scoliosis

1\,lmru basilar arachnoiditis ":::Vi1J'1l1m1L",ii'1l\,lr1\,l LLIii":::Vi1J~1ii hydrocephalus

1111~1nn~1

syringomyelia associate with tumor lJn":::Vi1J1\,lm!J~1nn~1 40 iJ lJn,,:::ml1l~lJ

,Q. . A • "" 'mmr'll'1l~ tumor ,,:::Vi1J~1~ dissociate sensory loss ",r'1l muscle wresting \,l'1llJn~1

syringomyelia associate trauma lJn,,:::ii progressive neurological deficit ~~,,:::, ,

d'l\,l1\,lr:::~1JVlIii1~"1nmr1J1I11L~1Ji1l"~LLm c:rU~lJ,,:::ij ascending myelopathy LL1J1J•unilateral

18

Radiologic evaluation

1. Plain film entire spinal and skull

ilth:;LtJ'lIu1un1~tJ<lnassociate lesion ",'1<letiology 'lI<l~syringomyelia mn

n~1H

lUn1~';j\j,,'iitJL'liUscoliosis, spinal canal widening, basilar invagination

2. MRI

LUUdiagnostic of choice lUn1~';j\j,,'iitJ syringomyelia, T1 weight 1\1~1Hl:jij,,'iitJ

morphology 'lI<l~cord LLi'l:;syringe till~ sagittal LLi'l:;axial planes 1\1~1~(l1(]extent

'lI<l~syringe till HI(] fibroglial septation till .. axial imge 1\1~1~m..i~tJ<lneccentricity

'lI<l~cavity LLi'l:;'lIU1~f1~1~tJ1~'lI<l~cord lu T1 weight, syringe ":;L~ULUUfusiformd

shape, well defined margin, low signal intensity L"'~<lUCSF

T2 weight ,,:;'Ii~tJlun1~1(]pathologic change lu cord L'liUtumor,

myelomalacia LLi'l:;n1~:j)~contrast ,,:; 1\1~1~mLtJngliosis ~<ltJ1 syringe ntJ tumor till

3. Ultrasound

iltJ~:;LtJ'Ilu1uintraoperation ",Ii~ laminectomy LoW<lYl":;localized LLi'l:;ll'ishunt

tube

4. CT myelogram

1\1m~(l';j\j,,'iitJ syringomyelia till"1nn1~ accumulation 'lJ<l~water soluble contrast

within the cord tJ~:;mru 4-12 i~L~~",Ii~:j)~il-ii<lLl\tJi'i<lcomulation radiation dose, false

negative (27%), LLu:;1111~1.•l1unHnyh MRI hi1il1

5. Dynamic imaging

LuuLVJf1\jf11mJ~Hlun1~';j\j,,'iitJnormal LLi'l:;abnormal CSF flow lu spine ~~m"

":;";11,xL-ii1hL~tJ~ntJmechanism 'lJ<l~n1nii~LLi'l:;'(~~U'll<l~cavity LLi'l:;'Ii~m~mntJn1~

l"m,;1L'liunHuil prominent flow lu cavity n1~l"n";1il1~tJshunt <l1",,:;1,xtJi'l~L~tJH

dynamic MRI technique

Management for syringomyelia

U"'llJU concept L~!J~ntJpathogenesis 'lI<l~syringomyelia !i~hji~L"U LLi'ldl

1\1m~r;h~1 ~yhl,xLii~~u~1 ~~t!Un1~~"1mnn1~l"n";1~~~untJ clinical LLi'l:;etiology

'lJ<l~syringomyelia

19

Syringomyelia associate hind brain herniationQ ••••• ...., d.ell 0

lJn1~~m~1V1mn~~~I'l'1ln1~Vl1craniovertebral decompression, syringe drainage,,

'I .."..•••C11

ventricular shunt ~\.ln~ruVllJhydrocephalus

Post traumatic syringomyelia

L~'1l~11IJtl"l.,UIJposttraumatic syringomyelia Lnl1l"l1narachnoiditis ~~i1n1~~1

posterior decompression Ll1Itln1n11laminectomy LL~:;intradural lysis of arachnoid

LLI1~creat meningocele L~'1lHfLijIJVI1~fluid bypass ~~U1"l:;1i1i~~~n~1n1~~1shunt

procedure

Syringomyelia saaociated tumor

Ll1Im~'~1un1~~i1 syrinx i~lJrilJ tumorw ad

infiltrate 'lI'1l~cord n1~mWlnl'l'1ln1~ resect tumor '1l'1ln

Anterior decompression• , d

Vl1~IJmru platibasia, basilar invagination ~1'1labnormality 'lI'1l~dens ~11i1itl1ltl

odontoid resection LLIJ:;u11m~ventricular shunt

Hydrocephalus associate hind brain herniation LLIJ:;U11~'1~ventricular shunt

Non surgical management

1. Contraindication to surgery; L'liIJrespiratory reserve compromise, severe limb

deficit

2. Stable condition

3. Spontaneous cure•4. Asymptomatic patients L~~1:;'1l1n1~"l:;progress "111

20

3.Topic review

Spinal dysraphrism

Definition dju~1~H'LL'r1Uf'111~~(1ItJn~'lJ'iNspinal cord ~Lii(1l"11nimproper

development (fusion) 'lJ'O~posteriot neuropore

Classification

1. open dysraphric malformation

myelomeningocele

hemimyelomeningocele

2. closed dysraphric malformation

lipomyelomeningocele

dermal sinus tract

myelocystocele

split cord malformation

nourenteric cyst

caudal agenesis

Embryology

1Um~~Ln"l:;L-ih1"1L~mrlUcongenital anomaly "l:;ilf'OwihhL~mrlu embryology, d •••••••• IV QI

'lJ'O~spinal cord n'Ou 'lI~\WII"l:;Iil'O~'r1~1Uf'l'Om~ development '1I'O~notochord LL~:;

primary, secondary neurulation. ,'1U\117 "1:;Lj-mnlillJnotochord process Ln(1l"l1nm~migrate '1I'O~ectoderm

L-ihltJ1uprimitive pit "'11~Lnlil m~I'l~1~ neural plate LL~:;vertebral bodies ;~m:;u~u, ,

.". '" Q"><II 4 '"'1 '" .••.m~YI"l:;I\n~ spinal cord nf'l'Odesignated neurulation 'lI~"l:;n~,mn(1lbrain LL~:;•

spinal cord "luli~ upper lumbar l'i~um:;u~Um~\\~1~'1I'O~spinal cord ~:;~U!h~"l1n,:ru

d'Jum:;u~um~ agglomeration '1I'O~cell, vacuolization, involution LL~:;retrogressive

differentiation ;~L1'Jn~1secondary neurulation ;~ primary neurulation "l:;L~~"l1n '1u

~ 21 li~ 25 I'i~u secondary neurulation "l:;Lnl1l'1u~25

- formation of vertebra ~mnI1l1u'1u~17 Ln(1l"l1nmesoderm,

Y1'8tJ lateral vi'fl•notocord "l:;\1U1~~LL~:;form d']u column oi'O~11u'1u~20 "l:;~~LLti~Ll'JU~1Cl-'Jnsomite ~~oi'O~1"l:;LU~!JumJ~~Ll'Jusclerotome n~1ml'Ju cartilage bone LL~:;ligament

21

, ,']J'1l~vertebra lii'1lhJ ~~'v1li'~"l1nVinotocord LLtJn"l1n ectoderm LL~::;endoderm

schrotome "l::;L.ji1hJ~'1llJ~'1ll.Jspinal cord LL~::;n~1mu••• vertebra 1ii'1l1u

1. myelomeningo~dF1'1labnormal exposure ']J'1l~segment of spinal cord in midline of back

.i'lI~ myelomeningocele LU'" most common form ']J'1l~spinal dysraphrism

pathogenesis

U"l~U"';J'II~1.tiJL~tJ1nl.Jn1~Ln,"2 '1l£h~A'1l

1. neural tube fail to close properly

2. close neural tube rupture open

~~'II~1.tiJLLm"l::;1i1rfl.Jm1lJL~'1lli'1llJ1nn~1t,"m~'1l~1 'Il1~1.J1~'1lth~L'li••• tunicamycin

'v11'1lvitamin a ~1;;l,"1"'({Iil'l'll,"~'1l~"l::;-ii1h'i neural tube 1~tI'" ci~"''II~1.tiJ ~ 2 L~'1l~1

vertebral cord closal sulci LU"'ci~•••~1.J1~'Il1~1.J1~'1lth~'1l1"l"l::;m::;iIi•••1,xLn," n1~']JtJ1tJlii'~']J'1l~•" .

central canal LLi\'~LLli1n'1l'1lnlJ1ci~"''Il1L'v1f;JVi-ii11,xLn,"L~'1l~1LU'" multifactorial ~~ genetic

LL~::;environment ~1.J~1'1l1~1.J1~'1lth~m::;~•••1,xLn," neural tube defect 1i1rL'li••• phenyltoin,

valpoic acid, viral infection LL~::;n1~']J1,"'Il1~'1l1'v11~1.J1~'1lth~L'li••• folic acid LL~::;zinc "l::;., . '

-ii11,xLn,"1i1r~~LL"'::;U11,x'v1~~Vi"l::;Iii'~F1~~Jln••• folic acid 0.4 mgld LY1'1l~,"'il1i1~1n1nn,"

spina bifida

Early detection

intrauterine detection dJ•••~~~'li~tJ1,xn1~Y1"l1~n.nn1~fm~n L'li••• terminate

pregnancy 'v11'1lllin1.t1nl.JyJ'1lLL~Lliin~~"l::;'Il1lJ1~nH' alphafetoprotien Lu•••Iii'~'li~tJ1i1rtl'ltJ"l::;, .

1,xL"l1::;afp 1"'({U,"1iVi 16-18 t'f1~~~'" "l::;;J accuracy 60-70% t'f1i~lJnl.Jn1nh

intrauterine us "l::;'Il~li~ 90-100% LLIiit'hAFP 'Il~LLIiiUS negative LL"'::;U11,xvi1• •,d • d

amniocentesis L~'1lI:J~::;l'Il.JAFP LL~::;acetylcholinesterase "l::;lJ accuracy 97%

Associate anomaly

ci~•••1'v1qjLLi\'~m1lJll,"un iii~~l.Ji~lJJi'~tJnA'1l nervous system ci~•••m1lJ 1ll'lun iii']J'1l~

~::;l.Jl.J~\l''l::;~l.J-.J'1ltJ~~ci~•••1'v1qj~~l.JlJmu ••• genitourinary system L'li••• hydroureter ci~•••

hydrocephalus ~1.J1Ji'li~ 80% m1lJll,"uniii~"'1 ~~1.J L'li••• chiari malformation,

22

Q , ,

micropolygyria cerebella dysgenesis, spine abnormality V1YiUU't}!JL'JIUanterior and

posterior fusion defect, hemivertebra

Clinical assessment

i1f't}~dl-l"l1n~general condition LLfl:;l-l1associate anomaly ~~'t}1"1"1:;i:ieJflj;J't}n1~

fm:t1'l~ ~~~~~iIf't}~yj"l1m.n'l~LLrii1'n~ru:;'1I't}~hydrocephalus L'liUHC, fontanelle,

movement '1I't}~LL'1IU'111'Ii1'n~ru:;LLfl:;~1LLmJ~'1I't}~lesion, associate anomaly of spine L'liU•scoliosis, kyphosis 'I moter LLfl:;sensory ~~'tl1"1"1:;~~1"1'l~!J1n'tl1"1"1:;Hfn1~'1Iej'uLL'1IU'1I1•Lri't}m:;~u pain LLfl:;lTn"l:;YiU~1i1urinary sphincter disturbance 'l~ti't}!J i1T~,rU"I:;iIf't}~~

i1'n~ru:; urine stream LLfl:;i1patulous anus ~1't}L1.M1LLfl:;m~"I:;~~1"1'1fluid U1L1ru•defect ~~!J ~1i1 infection l-l1't}hi

Radiologic evaluation

U"I'jUU radiologic evaluation ~Hfii~'t} US head Li1't}~hydrocephalus "I:;Hf CT

nnu follow up case n~rul'1l VP shunt l-l1't}i1neuro sign LLUfl~'Il1U film spine hj'li~!J

lUn1~ pre-op LYi~1:;~1spine L~nej'~hJt~LIJil-l~radiologic 't}U1~~U'l~LLri n1~'1•urinary tract

Treatment

- decision: -i~~tI~:;Mfl1.Un1~eh.r'lL~'t}fl'l retrograde meningitis LLfl:; preserve

neurologic function ~~,i1hii1 associate severe anomaly fI~~"I:;~1.r'l'VJn~1!JLYi11:;~1

survival rate 90%. normal intelligence 80% LLfl:;no assistant 85%

- time of surgery: LLU:;U1~1.r'lYlUYi1l1!Jlu24 i~hl~LL~mLj;Jmru~1LiJu"I:;~'t}1Mi~

72 i~Ll-l~ L'I!J rate infection hJLtI~!JULLtI\'I~mru~1.r'l-if1 LLU:;U11,x1ilCSF exam&culture••ri't}U3 flf~~1 hJi1 infection

1. preserve neurologic function

preserve neural tissue

preserve vascular supply

avoid inclusion dermoid

23

2. retethering LLl.I:;u11~yhpia to pia closure to tubular,~ ~

neural compressive during closure ---water tight closure LLI'II'I!l~J..Jspace Vi!l

~1'\.\ftJ

neural placode~ . ~

wound closure J..Jm:;VI1linear midsagittal closure ~1 mobilize muscle LL~:;

fascia mtJI111~riLLl.I:;u11~-.11•

hydrocephalus 10-20% 'JJ!l~~thtJllH1LiJlJIii!l~11'1shunt M~,r1.l11.1nnutl1

pre op hn~~ mark hydrocephalus ~~~:;-.111~wound healing hi~ hj~1LiJlJIi;!l~11'1VP

shunt L~tJ 1'l1J..J1~~~:;closure clinical 1111~~~lJn~:;Lnl117-14 1'l.l'\.\i\'~~1~111•post op care LLl.I:;u11~1.I!l1.l1'l~1~:;1'~CSF leakage ~1mL~~ motor LL~:;

sensory function m~~:;ni\'tJmL'\.\~!ll.l pre op 11.17-14 1'1.1long term follow up iIi!l~':J~ ,

urologic function, neurologic function V1LLtJ~~

occult spinal dysraphrism

tethered cord

pathophysiology L~!l':h neurological dysfunction 1l.1~thtJtethered cord

Lnl11~1n1 : impairment of oxidation metabolism Lnl11~1nspinal cord ischemia 'I111~

oxidation reduction ratio Lt.I~tJl.IhJATP ~111~~'I111~function 'JJ!l~neuron Cll11t.1n~2:

morpholigic change 11.1cord n~ru ~n~~m~~:;'1111~neuronal membrane ;;)n'JJ1111Lnl11

dysfunction Hi

LipomyeJomeningocele

Classification --- CHAPMAN: dorsal, terminal and transitional lipoma

Embryogenesis

dorsal and transitional lipoma

L~!l~1Lnl11~1ndefect 1l.1n1~closure 'JJ!l~neural lube during primary

neurulation 'I111~~n1~invasion tube 'I111~Lnl11lipoma Maclone L~!l~1Lnl11~1nd ,

premature dysjunction 'JJ!l~cutaneous LL~:;neuroectoderm Marin and padilla L'IIm1

Lnl11~1nI'l11J..JCll11t.1n~'JJ!l~paraxial mesoderm 1l.1n1~m:;~l.1n1~bending 'JJ!l~neural fold

LL~:;Melone L:n!l~1inner surface 'JJ!l~defect neural tube n~:;~1.I1~Lnl11fat ~1.1

24

: terminal lipoma

L~,;)~1Lncn'11nr1~1lJe1cntJn~'lI,;)~secondary neurulation L'W.n-'WI1I,;)'W'lI,;)~

retrogressive differentiation

Clinical feature

L'WLliimrnil neuro defect n';)'Wm!J 2 tJ '1:;~lJ midline ~1';)eccentric, ......'lumbosacral soft tissue mass neurologic defect m'1'1:;LLtnN 'lIru:;lJ activity L'Il'W';)';)n

ri1i~mu n~:;tcncn

,;)1n1~tJ~l1Il1'nd'J'Wpoor localize diffuse pain Vi leg, groin and perineum

m'1il back pain Hi, cutaneous manifestation ~lJ 80% L'II'Wcutaneous lipoma,

hemangioma, dermoid cyst, hypertrichosis, neurological defect ---asymetrical weakness

and patchy sensory loss, bladder and bowl function delayed or un successful toilet

training LL.JL'Winfant '1:;I1I~~'1ttli'tJ1n ti'10ml)'1:;~lJ urgency, frequency, post void

dribbing, foot deformity, hammer toes, vulgus or varus deformity

~1';) trophic change 'lI,;)~ skin ttli'

~m'1'1:;~lJlJ scoliosis

Radiology

MRI Li'J'Wstudy of choice '1:;~lJil high attenuation L'W T1 LLi'I:;

diminished signal L'W T2 film spine '1l1'1~lJ spinabifida, sacral agenesis, hemivertebra

I(Jscoliosis CT myelogram H'LLun terminal lesion '11n low transitional lesion

Treatment

LL'W:;U1L,xc.hiircn"(lnnu L~~1:;~~LLll'~1L'W'II~~LLm'1:;U~hJil neurologic defect. , ,LL.Jmm~l1'n'1:; progress mn;'WvhL,xLncn neurodeficit L'WVillcn~lJ~1ilu'1lulJ1nViLliinmu. .

-:;"'1 ., •••> 4 u mlJ neurologic intact

~,rnm~~1Iiicn 1. release cord tethering

3. preseNation of neural element

4. lipoma debulging

5. water tight dura and skin closure

25

Congenital dermal sinus

A'il tract ~flijl-llih!J stratified squamous epithelium ~UhlLL1.l'l midline ~~LLIii

occiput ~~ coccyx tC;)!J~uYi lumbar 41%, lumbosacral junction 35% tC;)!J~ tract

'1l1"l"l::'il~Lilr skin ~1'il~m;hhjL1.l intradural space

Embreology

LnC;)"l1nfailure of normal midline fusion ~::~~1~ ~tlC;)1-J~ 3-4 'I11L,xn ectodermd.~tI~ ~.J d J'~ ,~

~nC;)W1J1\\1.l neural tube ~C;)!JVlm1l-1l\n'1J'il~tract '1J1.lnudegree of seperation 1'l'l1.l~1.l

nnu dermal sinus 11.l lower sacral ~j.'il coccygeal LnW;nnfl'l1l-1Ejc;)tlniil'1J'il~u

canalization of tail bud tC;)!J~Tract "l:: associate I1U dermoid tumor 83%,

epidermoid tumor 13%

ClinicalA I " d "'" .o!lu~:;l-I1ru 75% "l::l-I1C;)'l!JCNS involvement 'lI~'1l1"HnC;)"l1ntethering "'~'il

, . ," d..Q' .••. ''I 'AcJlassociate tumor, 40% "l:;l-I1C;)'l!Jmeningitis 'lI~L'II'ilVl~UI'l'l1.l\~ry Lu1.lStaph aureus , E.

coli ",j.'il'1l1"l"l:;l-I11if'l!J purulent discharge "nn slinus 11if

Diagnosis

Hn1~1iI~'l~11~n1mtJ1.l",lin n1~H probe exam ~j.'il~C;)~ hi'Ji'l!J':iiJ"l'ilmLIii'1l1"l"l:;

'I111,xLnC;)n1~iilC;)L;'il11ifPlain film spine '1l1"l~U spina bifida, 11'l1.lRadiologic of choice

A'il MRI

Treatment

LL1.l:;~1L,xexcision dermal sinus Yi'il~L>lij'il sacrococcygeal level "1m1!J ~1'l~":;• •

tilM'il'ilm1~"'l-IM~'ll-l-.1~ intradermal portion "'1'il dermoid tumor

L1.ln~n:iI'l~~!Jmeningitis m~'I11 LP LLi\''lL,xantibiotic "l1.lfl~U LLi\''l~~ surgery

LLIii{hn associate abscess LL1.l:;~11,x'l11surgical drainage n'il1.lLLi\''l~~l-I1excision

sinus

26

Clinical,

,.h:;l-I1UJ 75% "l:;l-I11ir~tJCNS involvement ~~'1l1"lL1iI1l"l1ntethering ~1t1

associate tumor, 40% "l:;l-I11ir~tJmeningitis ;~b~tl~Vi1J~~1.IL~CIibtl1.l Staph aureus , E.

coli ",1'/l'/l1"l"l:::l-I1lir~tJ purulent discharge 'inn slinus llir

Diagnosis

HmTI1l1'l"111~mtJLiJ1.Iw<1'nmTH" probe exam ",1t1;;l11l~llJ'Ji~tJ7j'l"l,rtJLLvi'/l1"l"l:;

°1~- _.\'u- ,Vl1 mnl1ln1TI1ll1lL"lItlWI Plain film spine tl1"lVi1J spina bifida, i\~1.1Radiologic of choice

;)'/l MRI

Treatment,

bb1.l:;1l11,xexcision dermal sinus Yi'1l~L~ntl sacrococcygeal level '1n11!J mT"l:;~ .

••••••••• 0Ill

I1lI1l'/ltlnVl~"'l-Il1lT~l-IVI~ intradermal portion ~Ttl dermoid tumor

L1.ImtUMitJ meningitis FmVI1 LP bLi\'~L,xantibiotic "lUFlT1JLLi\'~~~ surgery

LLvi,hi1 associate abscess LLu:;1l11,x":i1surgical drainage n'/lU LLi\'1~~l-I1excision

sinus

• 27

Reference :

1. David G.Melone : Normal and abnormal early development of the nervous system,

Pediatric neurosurgery 1994 P.3

2. Donald H.Reigel : Spi~a bifida, Pechiatric neurosurg, 1994 P.51

3. Donald H.Reigel Tethered spinal cord, Pediatric neurosurg 1994 p.n

4. Robin P.Humphreys : Spinal dysraphrism, Neurosurgery 1996 P. 3453

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